Pathology often leads to death. This form of encephalitis is commonly diagnosed in women with ovarian teratoma.
Encephalitis is a group of brain diseases. Inflammation of the brain often develops due to the bite of an encephalitic tick, vaccination against a background of weakened immunity, infection. At the beginning of the 21st century, a new type of disease was discovered, which has an autoimmune etiology.
Interesting! The disease was first identified and registered in 2007 by scientists H. Dalmau.
Receptor encephalitis (anti-receptor encephalitis, anti-NMDA-receptor encephalitis) is an acute inflammation of the brain structures caused by an immune attack on the NMDA receptor. At the same time, their own immune cells “attack” the brain. The disease is severely treated, leading to dementia, dysfunction of the nervous system.
The disease affects the female gender, almost never occurs in men. Such women were identified deviations of a mental nature, confusion, intellectual problems. A high content of NMDA receptor antigens was also found. Pathology refers to a form of limbic encephalitis, which manifests itself in psychiatric disorders.
There are several stages of the disease:
- Prodromal The symptoms of a cold are manifested: fever, fatigue, pain in the head.
- Psychotic. The patient is tormented by unreasonable fears, depression, the person becomes withdrawn. There are problems with memorization, mental processes are disturbed. Crazy states, hallucinations of visual and auditory character develop. If you seek psychiatric help at this stage, the success of the treatment is guaranteed.
- Areactive. There is confusion, the patient has no reactions to verbal attitudes. Convulsive syndrome is increasing. A person can constantly smile.
- Hyperkinetic. Accompanied by involuntary motor acts, a person removes the jaw, clenches his teeth, reduces his eyes, waves his hands. Vegetative functions are impaired. The patient’s heart rate changes, pressure jumps, excessive sweating is recorded.
- Regressive. Recovery occurs under the condition that the pathology progresses no more than 2 months. The rehabilitation period is accompanied by amnesia.
The last stage does not occur in all cases. NMDA encephalitis is highly resistant to therapeutic methods, leading to death.
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Causes of pathology
The main causes of pathology include autoimmune attack of brain cells, the presence of tumor processes and infections. The pathological process begins to progress in a person at about 25 years of age, but there are cases of manifestation of the disease in 8-month-old babies.
Ovarian cancer was detected in 50% of cases. But many cases of encephalitis in the absence of oncology have also been reported. In children, the disease is detected without the presence of tumors.
In the children’s body, antibodies to NMDA receptors begin to actively replicate, blocking the activity of the receptors. After contact of antibodies with the receptor, neurons are damaged. This provokes the development of mental disorders, problems with motor function, epilepsy attacks. In many clinical cases, doctors cannot determine the specific cause of the disease.
How does the clinical picture
Initially, anti-NMDA-receptor encephalitis is manifested by flu-like symptoms that build up over several weeks. Many patients mistakenly turn to a psychiatrist who diagnoses bipolar disorder, schizophrenia. As a result, a person is treated incorrectly, which worsens his condition.
In children and adults, symptoms vary. There are minor violations of the neurological nature. The main symptoms of childhood disorders are behavioral problems, convulsive syndrome, psychiatric disorders.
As the disease progresses, paranoia, mental disorders, and seizures join. The patient moves the limbs strangely, there is a bizarre face grimace. Intellectual disturbances, dysfunction of memory, problems with speech are noted.
Fixed failure in the vegetative system. Respiratory failure, instability of arterial parameters develops. The patient may lose sensitivity.
Diagnosis of receptor encephalitis is complicated by the impossibility of its detection using magnetic resonance imaging. The results of the study do not show the presence of changes. Pathological process is detected only in a special mode. Then the lesions are found in the temporal region. Sometimes patients are referred to positron emission tomography, which detects cerebral cortex perfusion.
During puncture and examination of the obtained cerebrospinal fluid, minor changes are detected: an increase in the amount of protein and the concentration of immunoglobulin. Results of tests for viral infections are negative.
Note! Testing antibody titer-NMDA in cerebrospinal fluid and serum is used for correct diagnosis.
Many patients with receptor encephalitis have a brain tumor, which could be identified after the patient recovered. Therefore, oncologic screening is a mandatory diagnostic method.
An electrocardiogram, biochemical analysis of blood, feces and urine are used as concomitant diagnostic methods.
A person is identified in the intensive care unit, since pathology often leads to impaired respiratory functions. It is necessary to carry out artificial ventilation of the lungs.
After treatment for NMDA-encephalitis, the disease does not return for 7 years.
As therapeutic methods, such manipulations are carried out:
- Plasmapheresis. Purification of human blood.
- Immunotherapy. Intravenous immunoglobulin, glucocorticoid intake is required.
- The use of cytostatic agents.
- Use of anticonvulsive medications to eliminate seizures.
- The use of neuroleptics that control dyskinesias.
In the presence of a brain neoplasm, surgical intervention is required. Tumor inclusion affects the synthesis of antibodies that destroy nerve fibers. The faster the tumor is removed, the more likely the recovery.
Scientists are continuing to study the disease in order to invent effective treatment techniques that will make it possible to cure the patient more quickly. To do this, it is necessary to investigate the molecular structures involved in the process of reducing NMDA receptors.
Among the new drugs that supplement the main therapy, the substance Bortezomid, which effectively improves the patient’s condition in severe forms of pathology, is isolated. Starts to actively produce protein, as a result of which there is an enhanced synthesis of antibodies. Bortezomid reduces the concentration of antibodies that affect the progress of encephalitis.
Prognosis and complications
Diseases of autoimmune nature are difficult to diagnose. When an incorrect diagnosis is made, the patient may be referred to a psychiatric hospital, where therapy leads to the progression of mental disorders. Very often such patients fall into a coma. If a person ignores taking medication, vegetative disturbances appear, in 40% of patients death occurs.
As a result of the research, it was established that a favorable outcome of the therapy is observed provided that the neoplasms are eliminated within 4 months after their diagnosis. The first manifestations of receptor encephalitis bother a person for 2 months. The person is in a medical facility for 3 months.
After discharge from the clinic, patients report increased impulsiveness, problems with concentration. Some of the patients avoid the effects of the disease.
Often, autoimmune diseases cannot be completely cured. Treatment contributes to the prevention of progression of the pathology and the elimination of serious neurological complications. If receptor encephalitis is caused by a tumor process, then the removal of cancer guarantees the success of therapy.
Receptor encephalitis is a complex disease that requires competent therapy. Full recovery occurs with timely access to a professional specialist. Because the clinical picture of the disease is similar to many psychiatric disorders, the patient is often prescribed the wrong treatment. This leads to a worsening of the situation.
The following sources were used to prepare the article:
Bogadelnikov I.V., Bobrysheva A.V., Vyaltseva Yu.V., Chernyaeva Ye.S. Encephalitis caused by antibodies to NMDA receptors of neurons // Child Health Journal – 2013.
Malin D. I., Gladyshev V. I. Malignant neuroleptic syndrome or autoimmune anti-NMDA receptor encephalitis? Analysis of a clinical case with a fatal outcome // Journal of Social and Clinical Psychiatry – 2017.